An unusual presentation of juvenile systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a multisystem disorder with a diverse spectrum of clinical abnormality. Despite advances in our understanding of the disease, the aetiology and outcome remains unknown. We describe a 13-year-old boy who presented with paronychia, mouth ulcers and associated neutropenia. Appropriate investigations including a bone-marrow biopsy revealed a shift to the left, and in the absence of any other immunological abnormalities, a diagnosis of idiopathic neutropenia was made. The patient was successfully treated with granulocyte colony-stimulating factor but subsequently re-presented during the winter months with a purpuric facial eruption affecting his cheeks and ears. On histological examination of a biopsy taken from the ear, a microvascular thrombosis was seen. Laboratory investigations were positive for anticardiolipin antibodies, neutropenia and antinuclear antibodies (ANA), suggesting a diagnosis of juvenile (J)JSLE with antiphospholipid syndrome. This is an unusual presentation of SLE in a prepubertal white boy, with a malar and helical vasculopathy as a novel cutaneous presentation of JSLE.